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Malignant presacral ghrelinoma with long-standing hyperghrelinaemia

Falkmer, Ursula G.; Gustafsson, Thomas; Wenzel, Ralf; Wierup, Nils; Sundler, Frank LU ; Kulkarni, Harshad; Baum, Richard P. and Falkmer, Sture E. (2015) In Uppsala Journal of Medical Sciences 120(4). p.299-304
Abstract
Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A... (More)
Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A majority cell population displayed immunoreactivity to ghrelin, exceptionally with co-immunoreactivity to motilin. Somatostatin receptor scintigraphy and Ga-68-DOTATATE PET-CT demonstrated uptake in the metastatic lesions. High serum concentrations of total (desacyl-)ghrelin were found with fluctuations reflecting the severity of the symptoms. In contrast, the concentrations of active (acyl-)ghrelin were consistently low, as were those of chromogranin A (CgA).Conclusions. Neoplastically transformed ghrelin cells can release large amounts of desacyl-ghrelin, evoking an array of non-specific clinical symptoms. Despite an early dissemination to the skeleton, a ghrelinoma can be compatible with longevity after adequate radiotherapy. (Less)
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organization
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type
Contribution to journal
publication status
published
subject
keywords
Desacyl, acyl-ghrelin, ghrelinoma, hyperghrelinaemia, Lu-177 therapy, motilin, presacral carcinoid, skeletal neuroendocrine tumour, dissemination, theranostics
in
Uppsala Journal of Medical Sciences
volume
120
issue
4
pages
299 - 304
publisher
Taylor & Francis
external identifiers
  • wos:000365684900010
  • scopus:84945138508
ISSN
0300-9734
DOI
10.3109/03009734.2015.1054453
language
English
LU publication?
yes
id
2e675ab2-8453-457d-8613-d6439739f12e (old id 8525913)
date added to LUP
2016-02-08 07:22:15
date last changed
2017-01-01 03:20:42
@article{2e675ab2-8453-457d-8613-d6439739f12e,
  abstract     = {Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A majority cell population displayed immunoreactivity to ghrelin, exceptionally with co-immunoreactivity to motilin. Somatostatin receptor scintigraphy and Ga-68-DOTATATE PET-CT demonstrated uptake in the metastatic lesions. High serum concentrations of total (desacyl-)ghrelin were found with fluctuations reflecting the severity of the symptoms. In contrast, the concentrations of active (acyl-)ghrelin were consistently low, as were those of chromogranin A (CgA).Conclusions. Neoplastically transformed ghrelin cells can release large amounts of desacyl-ghrelin, evoking an array of non-specific clinical symptoms. Despite an early dissemination to the skeleton, a ghrelinoma can be compatible with longevity after adequate radiotherapy.},
  author       = {Falkmer, Ursula G. and Gustafsson, Thomas and Wenzel, Ralf and Wierup, Nils and Sundler, Frank and Kulkarni, Harshad and Baum, Richard P. and Falkmer, Sture E.},
  issn         = {0300-9734},
  keyword      = {Desacyl,acyl-ghrelin,ghrelinoma,hyperghrelinaemia,Lu-177 therapy,motilin,presacral carcinoid,skeletal neuroendocrine tumour,dissemination,theranostics},
  language     = {eng},
  number       = {4},
  pages        = {299--304},
  publisher    = {Taylor & Francis},
  series       = {Uppsala Journal of Medical Sciences},
  title        = {Malignant presacral ghrelinoma with long-standing hyperghrelinaemia},
  url          = {http://dx.doi.org/10.3109/03009734.2015.1054453},
  volume       = {120},
  year         = {2015},
}